IgG4-RELATED ORBITAL DISEASE: A CASE REPORT AND LITERATURE REVIEW

眼窩部IgG-4相關性疾病：個案報告與文獻探討

張瓊方(Chiung-Fang Chang) ； 吳鑑修(Chien-Hsiu Wu)

《中華民國眼科醫學會雜誌》 55卷 (2016/12) Pp. 36-44

https://doi.org/10.30048/ACTASOS.201612_55.0006

摘要

Purpose: IgG4-related disease (IgG4-RD), a chronic inflammatory disorder, is characterized by a high proportion of IgG4-positive plasma cells in the involved organs, which then causes fibrosis of these organs progressively. The etiology of IgG4-RD remains unclear; however, the possibilities of autoimmune and infectious agents have been hypothesized to trigger this disease. IgG4-RD of orbit (IgG4-ROD) was easily misdiagnosed as other entities. We report a case that was diagnosed as orbital pseudotumor at first, but later proved as IgG-4 ROD by pathological findings. In addition, recurrence at the same location of orbit was found after 15 months follow-up. Method: In this case, we report a male patient who presented with periorbital swelling with a detectable mass of the right eye at his first visit. After totally surgical removal of the orbital mass, the histopathological examination revealed IgG4-related orbital disease (IgG4-ROD). We discuss the associated clinical symptoms, hypothetic etiologies, differential diagnosis, laboratory results, imaging findings, and pathological findings, and treatments. In addition, we review the current literature on IgG4-ROD. Results: The mass was excised completely by using orbital exploration through a transcutaneous incision. The pathological results revealed dense lymphoplasmacytic infiltration, fibrosis, and a high proportion of IgG4-positive plasma cells. IgG4-ROD was diagnosed on the basis of the results of the histopathological examination of the tumor. The symptoms subsided after the mass was removed, and the patient was followed up regularly. But a recurrent orbital mass was found after 1 year of surgery, and mild upper gaze limitation was also noted. Conclusion: Although IgG4-ROD is a disorder diagnosed by pathological findings; it can be differentiated from mimic diseases through laboratory tests and imaging. IgG4-ROD was considered a benign disease; however, a risk of malignancy exists if the pathological findings showed a few reactive lymphoid follicles. Therefore, IgG4-ROD must be differentiated from the other malignant neoplasms.

關鍵字

IgG-4-related orbital disease ； IgG-4 ； pseudotumor ； autoimmune pancreatitis (AIP)

並列摘要

目的：IgG4相關疾病是一個慢性發炎性疾病，其特色是在受影響的器官中可發現廣泛浸潤的IgG4漿細胞以及纖維化組織。此疾病的病因還尚未清楚，但目前認為自體免疫活性和感染病原菌為可能的誘發因子。方法：我們報告了一個男性病患案例，主訴是右眼眼周腫脹約半年合併複視情形，根據術後病理學組織切片診斷為IgG4眼窩相關疾病。並且，我們蒐集整理了相關文獻，從臨床症狀、病因假說、鑑別診斷、檢驗數據、影像檢查、病理切片和治療等層面去探討此疾病。結果：此病患接受經皮下切口腫瘤切除手術，病理切片顯示淋巴球與漿細胞球浸潤，組織纖維化以及廣泛IgG4陽性漿細胞球浸潤。術後半年病人傷口復原良好，眼周腫脹及複視症狀顯著改善。然而，追蹤一年後發現復發之眼窩腫瘤，但因無影響外觀和視覺，目前藥物治療中。結論：雖然IgG4相關眼窩疾病是依照病理學診斷之疾病，我們仍然可以從抽血檢驗或影像學檢查去鑑別診斷。治療方式目前有手術切除，全身性類固醇施打，免疫抑制療法等。雖然IgG4相關疾病目前認為是預後良好的疾病，但仍須排除惡性腫瘤的可能。